CEBPA-bZip mutations are associated with favorable prognosis in de novo AML: a report from the Children’s Oncology Group

Tarlock K, Lamble AJ, Wang YC, Gerbing RB, Ries RE, Loken MR, Brodersen LE, Pardo L, Leonti A, Smith JL, Hylkema TA, Woods WG, Cooper TM, Kolb EA, Gamis AS, Aplenc R, Alonzo TA, Meshinchi S. CEBPA-bZip mutations are associated with favorable prognosis in de novo AML: a report from the Children’s Oncology Group. Blood. 2021 Sep 30;138(13):1137-1147. doi: 10.1182/blood.2020009652. PubMed PMID: 33951732; PubMed Central PMCID: PMC8570058.

Patients with biallelic mutations in CEBPA have improved survival, and this genotype is classified as favorable in the European Leukemia Net and National Comprehensive Cancer Network guidelines. In a study of over 3000 patients in Children’s Oncology Group (COG) studies of acute myeloid leukemia (AML) combining clinical and genetic analysis, Tarlock et al identify monoallelic mutations in the C-terminal basic leucine zipper (bZip) of CEBPA to be prognostic. They report that single bZip and biallelic CEBPA mutations have identical prognoses, suggesting that in young patients with AML, these entities should be considered equally favorable.